一例真性毛细胞白血病病例的免疫及分子生物学鉴定

Immunological and molecular biological identification of a true case of T-hairy cell leukaemia.

作者信息

Demeter J, Pálóczi K, Földi J, Hokland M, Hokland P, Benczúr M, Lehoczky D

机构信息

National Institute of Haematology and Blood Transfusion, Budapest, Hungary.

出版信息

Eur J Haematol. 1989 Oct;43(4):339-45. doi: 10.1111/j.1600-0609.1989.tb00309.x.

DOI:10.1111/j.1600-0609.1989.tb00309.x

PMID:2573540

Abstract

A hairy cell leukaemia (HCL) patient is presented in whom the peripheral blood mononuclear cells (PBMCs) carried suppressor T-cell markers (CD3+, CD2+, CD8+/CD4-, CD38+). Analysis of genomic DNA of PBMNC showed the presence of a monoclonal population of T cells, the T-cell receptor (TCR) beta-chain gene being rearranged on both alleles (DR/DR), while the immunoglobulin (Ig) heavy chain-genes were in germline configuration. The neoplastic cells were found to react with the monoclonal antibody RAB-1 - originally described as belonging to the B lineage-restricted monoclonal antibodies - and to carry RAB-1/CD-8 in a double marker assay. Natural killer activity of PBMNCs against K562 target cells was severely reduced, while the cells were found to exert strong antibody-dependent cellular cytotoxicity.

摘要

本文报告了一名毛细胞白血病（HCL）患者，其外周血单个核细胞（PBMC）带有抑制性T细胞标志物（CD3 +、CD2 +、CD8 + / CD4 -、CD38 +）。对PBMC的基因组DNA分析显示存在单克隆T细胞群体，T细胞受体（TCR）β链基因在两个等位基因上均发生重排（DR / DR），而免疫球蛋白（Ig）重链基因处于种系构型。发现肿瘤细胞与单克隆抗体RAB - 1发生反应 - RAB - 1最初被描述为属于B谱系限制性单克隆抗体 - 并且在双重标记试验中携带RAB - 1 / CD - 8。PBMC对K562靶细胞的自然杀伤活性严重降低，而发现这些细胞具有强大的抗体依赖性细胞毒性。